Physiological and pathological functions of PQBP-1
Project/Area Number |
12670596
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Research Category |
Grant-in-Aid for Scientific Research (C)
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Allocation Type | Single-year Grants |
Section | 一般 |
Research Field |
Neurology
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Research Institution | The University of Tokyo |
Principal Investigator |
OKAZAWA Hitoshi Univ. Tokyo, Dept. Neurology, Res. Associate, 医学部・附属病院, 助手 (50261996)
|
Project Period (FY) |
2000 – 2001
|
Project Status |
Completed (Fiscal Year 2001)
|
Budget Amount *help |
¥3,300,000 (Direct Cost: ¥3,300,000)
Fiscal Year 2001: ¥1,100,000 (Direct Cost: ¥1,100,000)
Fiscal Year 2000: ¥2,200,000 (Direct Cost: ¥2,200,000)
|
Keywords | PQBP-1 / polyglutamine / transcription / cell death / neuron / spinocerebellar degeneration / ポリグルタミン / 神経細胞死 / U5-15kD / スプライシング / スプライミング |
Research Abstract |
In this project, we have analyzed physiological and pathological functions of PQBP-1, which we found as a novel binding protein to the polyglutamine sequences. First, we found that PQBP-1 also interacts with ataxin-1, a causative gene product of SCA1, and induces transcriptional repression cooperatively. The interaction between PQBP-1 and ataxin-1 inhibits transcription not in the inclusion body but in the nuclear matrix, suggesting a new cascade of pathogenesis. Furthermore, our study showed that the interaction leads to reduction of phosphorylated form (=active form) of RNA polymerase II. These results, together with transgenic mouse of PQBP-1, will contribute for understanding this type of neurodegenerative diseases.
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Report
(3 results)
Research Products
(12 results)
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[Publications] Okazawa H.. Rich T., Chang A., Lin X., Waragai M., Kajikawa M., Enokido Y., Komuo A., Kato S., Shibata M., Hatanaka H., Mouradian M.M., Sudol M., Kanazawa I.: "Interaction between mutant ataxin-1 and PQBP-1 affects transcription and cell death."Neuron. (in press). (2002)
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